Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal, developmental lung disorder, which usually presents as persistent pulmonary hypertension of the newborn (PPHN) unresponsive to treatment. The authors present their own experience with three cases admitted during the last 15 years. 1. Introduction Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV; OMIM number 265380) is a rare, fatal, developmental lung disorder, which usually presents as persistent pulmonary hypertension of the newborn (PPHN) unresponsive to treatment [1, 2]. The majority of the reported cases have been associated with other multiple congenital nonlethal anomalies, most frequently involving the cardiovascular, gastrointestinal, urogenital, and musculoskeletal systems [1]. Increasing awareness of this clinical entity may prevent the use of more invasive and futile treatments, including extracorporeal membrane oxygenation (ECMO). We present three cases of ACD/MPV associated with gastrointestinal and urological malformations. All the newborns had an overwhelming course, with PPHN and hypoxemia refractory to treatment. The diagnosis of ACD/MPV was established by autopsy. 2. Cases Report 2.1. Case Report 1 Full-term female newborn admitted to our NICU (Neonatal Intensive Care Unit) in 1997 was diagnosed with severe bilateral hydronephrosis. She was the second daughter of a young healthy, unrelated couple. On prenatal ultrasounds severe bilateral hydronephrosis and oligoamnios were detected. Delivery occurred by C section at 38-week gestational age. The Apgar score was 5/8 and birth weight 3170？g. During the first hours of life patient developed increasing respiratory distress, with hypoxemia and bradycardia, and was intubated and ventilated. Chest radiograph showed a mild haziness pattern and echocardiogram excluded structural heart disease and demonstrated signs of PPHN. At hour 30 of life a rapid clinical deterioration was observed, with refractory hypoxemia and persistent severe metabolic acidosis, despite ventilatory optimization. Antibiotic therapy with ampicillin and gentamicin was administered since admission to NICU and was later adjusted to ampicillin, cefotaxime, and amicacin. Septic workup was negative, including blood, urine, and cerebrospinal fluid cultures. The planned renal ultrasound and voiding cystourethrography (VCUG) were not preformed since the patient’s clinical condition deteriorated rapidly and newborn died on the second day of life. On autopsy severe bilateral hydronephrosis was confirmed and signs
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